Prenatal Marfan syndrome: report of one case and review of the literature.

OBJECTIVES: Our objective was to describe the features of prenatal Marfan syndrome. METHODS: Doppler fetal echocardiograms were performed. The morphology and rhythm of the fetal heart were examined sequentially. RESULTS: The case was referred because of cardiomegaly and dilated great vessels. Sequential Doppler echocardiographic evaluation led to the diagnosis of prenatal Marfan syndrome. The main features are cardiomegaly, dysplastic atrioventricular valves with tricuspid regurgitation and dilated great vessels, which can be aneurysmal at their origin. The fetus died in utero at 39 weeks of gestation because of cardiac failure. Pathological study confirmed the Marfan habitus and complications. Molecular genetic study showed a de novo point mutation in exon 26 of the FBN1 gene. CONCLUSION: We report a case of prenatal Marfan syndrome diagnosed by sequential evaluation of the cardiac signs, which are essential for prenatal diagnosis. The prognosis seems as poor as the neonatal one. The prenatal diagnosis is essential for adequate counselling.

[Intraperitoneal hemorrhage from rupture of uterine varicose vein during pregnancy: case report and review of the literature]. / Hémopéritoine par rupture de varices utérines en cours de grossesse.

We report a case of spontaneous rupture of uterine varicose vein revealed by a massive peritoneal hemorrhage in a 34-year-old patient at 27th weeks of gestation. Laparotomy enabled diagnosis and treatment of this complication. The pregnancy was continued without any problem with a normal delivery at term of a healthy child.

[Bowel obstruction revealing post-traumatic diaphragmatic hernia in a pregnant woman]. / Syndrome occlusif chez une femme enceinte révélant une hernie diaphragmatique post-traumatique méconnue.

We describe a case of late post-traumatic diaphragmatic hernia revealed during pregnancy by bowel obstruction. The diagnosis was made during exploratory laparotomy at 29 weeks. After surgical repair of the hernia the pregnancy went to term uneventfully. A healthy baby was delivered at 39 weeks by elective cesarean section.

[Delayed delivery in twin pregnancy]. / Accouchement différé d'une grossesse gémellaire.

We report the observation of a primigravid patient, presenting a spontaneous twin pregnancy bichorionic and biamniotic. After expulsion of the first twin at 20 weeks and two days, expectancy is adopted and the second twin is born at 31 weeks and three days. In front of this not so frequent obstetrical situation and the absence of consensus, we discuss three points: cerclage, tocolysis and the prophylactic antibiotherapy. We specify that the attempt at differed childbirth is aimed at improving the foetal prognosis of the second twin by bringing it into the viable term, as well as obtaining a survival with the least possible number of sequels.

Sepsis due to Clostridium perfringens after pregnancy termination with feticide by cordocentesis: a case report.

We report a case of sepsis due to Clostridium perfringens after termination of pregnancy at 22 weeks with feticide by cordocentesis. Three weeks earlier, the 41-year-old patient had undergone an amniocentesis and a full trisomy 13 karyotype had been discovered. Feticide was performed by injection of thiopental and potassium chloride after percutaneous umbilical foetal blood sampling through the same needle. The patient delivered vaginally with signs of chorioamnionitis and septicaemia. She recovered under broad-spectrum antibiotherapy. C. perfringens was present in maternal blood cultures, placental smears and foetal organs. We discuss the possible mechanisms of infection by C. perfringens, including inoculation of intestinal germs.

Effects of maternal dexamethasone administration on fetal Doppler flow velocity waveforms.

OBJECTIVE: To investigate the effects of maternal dexamethasone administration on umbilical and fetal cerebral artery flow velocity waveforms. DESIGN: Cross-sectional study. SETTING: Department of Obstetrics and Gynaecology, Robert Ballanger Hospital, Aulnay-sous-Bois, France. SAMPLE: Twenty-six pregnant women with singleton pregnancies considered at risk for preterm delivery. At baseline, all pregnancies had normal fetoplacental vascular resistance. METHODS: These women were given weekly six intravenous doses of 4 mg of dexamethasone eight hours apart. MAIN OUTCOME MEASURES: Doppler studies were performed from both umbilical artery (UA) and fetal middle cerebral artery (MCA) before (day 0), during (day 2), immediately after (day 4) and shortly after (day 7) every steroid course. RESULTS: No significant variation was noted in both umbilical artery pulsatility index (PI) and fetal heart rate through dexamethasone therapy. Compared with mean initial values, we found on day 4 a significant decrease in MCA PI of 0.28 (F = 7.17, P < 0.001) and a significant increase in UA:MCA PI ratio of 0.08 (F = 3.85, P = 0.013); in contrast no significant change was documented on days 2 and 7 in both MCA pulsatility index and UA:MCA PI ratio. After multiple regression analysis, only the decrease in fetal middle cerebral artery pulsatility index on day 4 remained significant (F= 5.84, P= 0.001). CONCLUSIONS: The current study finds in healthy fetuses a transient, significant and unexplained decrease in fetal middle cerebral artery impedance on the fourth day following maternal dexamethasone administration. Further basic research and clinical studies including larger sample sizes or pregnancies with fetoplacental dysfunction are needed.

Absence of flow velocity waveform changes in uterine arteries after bilateral internal iliac artery ligation.

A case is reported of bilateral internal iliac artery ligation during cesarean delivery for intractable hemorrhage. Uterine artery Doppler flow velocity waveforms were documented before and after the procedure. After the ligation the uterine arteries could still be visualized in the appropriate anatomic location, and no changes in Doppler flow velocity waveforms were documented.

Perinatal mortality in twin pregnancies: a 3-year analysis in Seine Saint-Denis (France).

OBJECTIVES: Our purpose was to evaluate perinatal mortality in twins and to investigate factors associated with this mortality. STUDY DESIGN: A prospective study on the perinatal mortality was performed in the department of Seine-Saint-Denis. Out of all the perinatal deaths, we have retrospectively isolated those arising from twin pregnancies. RESULTS: There were 54 twin pregnancies complicated with perinatal death. The perinatal mortality rate in twin pregnancy was 78.0 per 1000 twin babies delivered. Out of 86 twin deaths, 38 (44.2%) were born before 28 weeks gestation and out of 82 twin perinatal deaths, 37 (45.1%) weighed less than 1000 g. Chorionicity was recorded in 44 twin pairs: 21 (47.7%) were dichorionic and 23 (52.3%) monochorionic. Finally, out of 48 twin sets there were four (8.3%) monoamniotic pregnancies. CONCLUSIONS: The present data show that extreme prematurity represents nearly half of perinatal mortality in twins. This study indicates also a significant proportion of monochorionic placentation among twin pregnancies with poor outcome.

[Inherited factor VII deficiency and pregnancy. Apropos of 1 case]. / Déficit constitutionnel en facteur VII et grossesse. A propos d'un cas.

Hereditary factor VII deficiency is a rare autosomal recessive condition. Factor VII's level elevates during pregnancy in normal patients, as well in deficient individuals for some authors. Various treatments (fresh frozen plasma, prothrombin complex or factor VII concentrate) have been used to lessen the peri-partum hemorrhage in those factor VII-deficient pregnant women. We report the case of a pregnant woman presenting a factor VII deficiency (level 4%), without variation of level during her pregnancy. The single infusion of factor VII concentrate, prior to delivery, has elevated factor VII's level at 17% and has likely permitted minimal post-partum bleeding. The peripartum management of factor VII deficiency is discussed.

Detection and measurement of fetomaternal hemorrhage following diagnostic cordocentesis.

OBJECTIVE: The aim of this study was to investigate the effect of cordocentesis on fetomaternal hemorrhage (FMH). METHODS: One hundred and three diagnostic cordocenteses, without any other associated procedure, were performed at 23-40 weeks' gestation. FMH was detected using the Kleihauer-Betke staining of maternal blood taken immediately before and after cordocentesis. RESULTS: Significant FMH occurred after 40 (38.8%) of the 103 procedures. An increased risk of fetal bleeding was associated with both an anterior placenta (odds ratio (OR) 5.89; 95% confidence interval (CI) 2.27-15.3; p < 0. 001) and a transplacental cordocentesis (OR 37.0; 95% CI 2.15-636; p < 0.001). The volume of FMH was greater after cordocentesis with an anterior placenta (90th percentile 6.20 ml) than after cordocentesis with a lateral (90th percentile 4.58 ml) or posterior placenta (90th percentile 1.35 ml) (p < 0.001). After fetal blood sampling, significant FMH occurred more frequently with a procedure duration of 3 min or more (OR 4.45; 95% CI 1.70-11.7; p = 0.002) and with two or more needle insertions (OR 4.65; 95% CI 1.80-12.1; p = 0.001). CONCLUSION: FMH following cordocentesis may be related to placental injuries. This event is influenced by placental location, procedure duration and the number of needle insertions.

Changes in umbilical artery, middle cerebral artery, and aorta blood flow Doppler waveform pulsatility indices after funisocentesis.

This study evaluates the effect of funisocentesis on umbilical artery, fetal cerebral artery, and aortic circulation. The pulsatility index in the umbilical artery, fetal middle cerebral artery, and descending aorta was measured by pulsed Doppler ultrasonography before and after 41 diagnostic funisocenteses. Percutaneous umbilical artery blood sampling was associated with a significant decrease in umbilical artery pulsatility index (mean -0.132, standard deviation 0.259, P = 0.002) and in middle cerebral artery pulsatility index (mean -0.143, standard deviation 0.260, P = 0.001). The decline in resistance to flow of the umbilical artery (r = 0.340, P = 0.029) and middle cerebral artery (r = 0.457, P = 0.002) was correlated with gestational age at sampling. These findings suggest that alterations in the waveforms from both the umbilical and the fetal cerebral circulations can be induced by fetal blood sampling.

[Predictive value of cervical-vaginal prolactin in the evaluation of premature labor risk]. / Valeur prédictive de la prolactine cervico-vaginale dans l'évaluation du risque d'accouchement prématuré.

OBJECTIVES: To determine whether the presence of cervico-vaginal prolactin during pregnancy is significantly associated with preterm delivery. STUDY DESIGN: A cohort of 64 pregnant women between 21 and 34 weeks of amenorrhea underwent a washing of the exocervix and vaginal fornices with a normal saline solution. The fluid was then aspirated and centrifuged. Samples were stored at -70 degrees C and later prolactin level was determined by radioimmunoassay. Test was considered as positive for a prolactin concentration higher than 2 ng/ml. Statistical analysis were realized by Student's t test, Fisher's exact test and chi 2 test. RESULTS: In patients with preterm labor, positive cervico-vaginal prolactin had a positive predictive value of 36% and a negative predictive value of 94% for a preterm delivery before 34 weeks of gestation (respectively 45% and 79% before 37 weeks). The sensitivity of a positive test was 31% for preterm delivery before 37 weeks of gestation and specificity was 87% (respectively 57% and 88% before 34 weeks). Patients with a positive prolactin test had a significantly shorter latency between testing and delivery (33.7 days vs 52.4 days; p < 10(-9)). No delivery occurred during the following weeks for patients with a negative prolactin test and, among those, only one delivery occurred during the second week following the test. Positive prolactin tests correlated with a mean cervical dilatation of 1 centimetre at the time of testing, while it was of 0.6 centimetre for patients with a negative prolactin test. CONCLUSIONS: Cervico-vaginal prolactin seems to be a non convincing marker for preterm delivery but indicative of a shorter latency from testing to delivery in symptomatic patients. Further investigations are necessary to evaluate accuracy of cervico-vaginal prolactin as a biochemical marker for imminent delivery in patients with preterm labor.

Ominous normalization of middle cerebral artery flow velocity waveforms preceding fetal death: case report.

The loss of middle cerebral artery compensatory vasodilation appears to be ominous in fetuses with absent end-diastolic umbilical waveforms. The authors report a case with a loss of the 'brain-sparing effect' 24 h before fetal death. Current pathophysiological explanations are discussed.

[Doppler velocimetry in the umbilical, middle cerebral and aortic arteries, before and after cordocentesis]. / Vélocimétries Doppler des artères ombilicales, cérébrales moyennes et aortiques, avant et après cordocentèse.

OBJECTIVE: Prospectively evaluate the effect of cordocentesis on the umbilical, fetal cerebral and aortic circulation. METHOD: Fetal blood was sampled for diagnostic purposes in 21 pregnant women at 21 to 38 weeks gestation. Ten patients undergoing amniocentesis served as controls. The resistance index (RI) in the umbilical and middle cerebral arteries and the mean blood velocity (Vm) in the descending aorta were measured with pulsed Doppler before and after blood sampling. Variations in umbilical and cerebral RI and in aortic Vm were recorded. RESULTS: There was a significant drop in both umbilical RI (mean +/- SD = -0.049 +/- 0.078; p = 0.009) and middle cerebral RI (-0.077 +/- 0.058; p < 0.0001) after cordocentesis. The drop in umbilical RI was greater when the second Doppler measurement was made early, when the blood was sampled transplacentally and in early gestational age. Reduction in fetal cerebral artery RI was also greater for transplacental puncture. The fetal descending aorta Vm did not change significantly after blood sampling. There were no variations in Doppler index before and after amniocentesis. CONCLUSIONS: Changes in blood flow velocity waveforms as measured by pulsed Doppler in the umbilical and fetal cerebral arteries can be induced by fetal blood sampling. Decreased resistance in the placenta and fetal circulation would imply release of nitric oxide.

[Congenital auriculo-ventricular block and anti-Ro (SS-A)/anti-La (SS-B) antibody]. / Bloc auriculo-ventriculaire congénital et anticorps anti-Ro (SS-A)/anti-La (SS-B).

Congenital heart blocks due to immunological causes are rare. A case is reported of a fetus with auriculo-ventricular block diagnosed at 22 weeks of amenorrhoea and intrauterine death at 32 weeks. The authors discussing the case find the most likely link: an anti-RO (SS-A) and anti-LA (SS-B) immunological block and they suggest that there are minor localised lesions in the nodal tissue which gives rise to benign disturbances of cardiac rhythm and they point out ways of preventing intrauterine auriculo-ventricular block.

[Malignant mixed mesodermal tumor detected by uterine inversion. Apropos of a case. Review of the literature]. / Tumeur mésodermique mixte maligne révélée par une inversion utérine. A propos d'un cas. Revue de la littérature.

The authors report the anatomoclinical observation of an 82-year old patient presenting a mixed malignant mesodermal tumour (MMMT) revealed by a uterine inversion. The histological examination revealed sarcomatous and carcinomatous lesions with zones of chondroid metaplasia infiltrating largely the myometrium. A total vaginal hysterectomy, accompanied by radiotherapy, got the better of an early local relapse. The histogenetic hypotheses of MMMTs, the particular circumstances of the tumour revelation, as well as the methods of treatment are reviewed.